Comparison of clinical features of alveolar RMS with pax-fkhr fusion transcripts and their prognostic impact

S. Stegmaier; K. L. Schaeffer; C. Poremba; B. Kazanowska; E. Hallmen; I. Haug; J. Treuner; I. Leuschner; E. Koscielniak

doi:10.1055/s-2004-828596

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Klin Padiatr 2004; 216 - 51
DOI: 10.1055/s-2004-828596

Comparison of clinical features of alveolar RMS with pax-fkhr fusion transcripts and their prognostic impact

S Stegmaier ¹, KL Schaeffer ², C Poremba ², B Kazanowska ³, E Hallmen ¹, I Haug ¹, J Treuner ¹, I Leuschner ⁴, E Koscielniak ¹

¹Olgahospital, Stuttgart, Germany
²Uni-Klinikum Düsseldorf, Germany
³Univ. Children's Hosp. Wroclaw Poland
⁴Path. Institut Kiel, Germany

Congress Abstract

The translocations t(2;13)(q35;q14) and t(1;13)(p36;q14) are associated with alveolar RMS, generating the chimeric genes pax3-fkhr and pax7-fkhr. To analyse their clinical relevance we examined tumor samples from pts registered in the CWS-86, -91, -96-studies by RT-PCR. We compared clinical characteristics and prognosis for 75 alveolar RMS pts with a detected translocation. 59 tumor samples were positive for pax3- (78%) and 17 for pax7-fkhr (22%). The distribution by postsurgical stage, age and TN characteristic in pts with these pax-transcript-types was similar. The EFS at 3yrs for all pts analysed with primary localised tumors was 34% (pax3 24%, pax7 10%; p=0,31). Out of 30 (36%) metastatic pts 11 were alive in 1.CR in the pax3 and 3 out of 5 in the pax7 group (p=0,35). The fusion type seems not to correlate with prognosis in pts with alveolar RMS.